Hello all, I am in the hospital riding out an attack. My first and only attack was last year almost to the day. I have been on a treatment of theyophilline 400mg 2x a day Ivigg 50g once every 3 months. This current attack seems to have been triggered by the same upper respritory virus I had last year.The good news is this attack is progressing much slower and my doctors know what they are battling this time. My hemoglobin is slowy rising. It is now at 18.2. It was 13.8 Thursday morning. My last attack ( resulting in faciotomies on all 4 limbs) escalated in a 12 hour period. I am well into 24 hrs with this attack. My legs are swollen and I can no longer sit up or stand without discomfort, light headedness, and nausea. Since being in the hospital and consulting with Dr. Druey, I have received 125g of ivigg, steroids, but no other fluids. My potassium is very low and my kreatine levels are too high for the Nephrologists comfort.My heart rate is high due to extra theophylline dose. So no ivigg today, just monitoring blood pressure, hemoglobin levels, drinking only 1 liter of fluids, and hoping that the recruitment phase begins soon and this does not progress to an acute attack like my last one. I know we all present differently, but I am wondering if any one has been able to avoid an acute attack once the progression has started? I feel like we are somewhat ahead of the curve this time due to the fact that I had some ivigg in my system. I will obviously have to move to monthly treatments during flu season or all the time. If anyone has a similar experience and can shed a little light that would be greatly appreciated. Trying to remain calm and hoping for a good outcome. Cristina

Dear Cristina: We wish you all the best in riding out this episode. In terms of minimizing or even aborting it, the best ways we know of are to give patients (a) steroids intravenously (Methylprednisolone 125 mg IV every 3-4 hours, see Disorder Treatments, in the Disorder Details section of this site) and (b) IVIG, as you are getting. Once this episode is over, your doctors will hopefully be persuaded that your previously reported regimen of daily Theophylline and IVIG every 3 months is NOT working, and they will transition you to the standard one of monthly infusions of IVIG and no Theophylline.

Cristina - Your account of your present attack sounds so much like what I've experienced so many times. As to your question regarding success in lessening the severity of an episode - I've been on monthly IVIg for a little over two years now. I've had one episode while on IVIg - about a year ago and I was a little over a week late getting my infusion because I was trying to get some dental work done. I ended up getting the dose in the hospital and I was sent home after only four days as compared to two weeks per each previous episode (pre-diagnosis). I went home with oxygen which I needed only for a week. That was the only time I had been in the hospital where the doctors actually knew what I had so they were more comfortable letting me go home when I felt like I was able. That attack was not nearly as severe as the previous ones and again - I now know not to get the dose late and not to put off even the most insignificant seeming dental work! (Also - I stopped taking theophylline about two years ago as well, so it was not a factor.) I hope things go better for you this time around. Really, really hope you get better in time to have a great holiday! I'm saying a prayer for you! Carole T.

Hi all, Thank you for the reponse. I seem to be entering the recruitment phase. My hemoglobin maxed out at 18.9. I am now back to 14.2. My kidneys are starting to pull off fluid. The doctors ended up giving 500cc's of fluid after waiting 48 hrs. over a 5 hour period to get my blood from being too tacky and keep my kidneys working. That was the turning point. My heart rate and blood pressure are back to nomal, and kreatinine and potassium leveled out. I have been trying to make every 3 months of Ivigg work, but I see that it is too risky. It is hard to admit that I have this awful disease but am ready to do what it takes to treat it. I am comforted in the fact that my team of doctors did everything correct this time around. I believe more and more education on CLS is saving our lives. I hope to be out of the hospital in 2 more days. All the best, Cristina

Thanks for the update and good luck, Cristina! As mentioned previously, your doctors will hopefully be persuaded that your prior regimen of daily Theophylline and IVIG every 3 months is NOT working, and they will transition you to the standard one of monthly infusions of IVIG and no Theophylline. Try to get them to obtain pre-approval for such a regimen NOW from your insurance company, while this episode is still fresh in their minds. Once you are discharged, unless you've got a really competent and compassionate personal doctor, you'll be on your own.

Hi Cristina , I have been my brother allen's advocate with his scls journey for the past 9 yrs. His first attack was similar to yours(4 fasciotimies)and up until that point he worked out at the gym several times a week which is what help save his life. A little over a year after his first attack he had dabbled a little with reducing or occaisonally skipping the theophylline that mayo clinic had recommended(they weren't using ivig in the us at that time) and he ended up with his second episode which sounds very much like yours. He has managed to get by only taking theophylling to date and occasionally has an er visit which they manage using the protocol that arturo mentioned above and they will give him judicious fluids, prednisone, sometimes albumin and his theophylline and then alot of wait and see. Arturo and dr druey have always been extremely helpful and I am eternally grateful. Knowledge is power and sounds like u have a good grasp on this but I would encourage you to be proactive as arturo mentioned because the best way to manage this disease is to prevent the episodes from occurring. In addition to drug therapy make sure you get your rest, stay hydrated and take care of you.I am happy that you are in the recruitment phase and wish you all the best. Linda

Hello Christina. I'm glad things seem to be turning around, and I hope you continue to recover. Your experience is very much like my husband's (first attack resulted in 4-limb compartment syndrome requiring fasciotomies). Like you did, he receives IVIG in the ER when he had an attack, and it turns things around fairly quickly for him (4 days in hospital compared to 6 months for the first episode). He also now gets IVIG monthly which has reduced his attacks by 50%. We feel the monthly treatments are worth the effort. Feel better soon!

I am still in the hospital, but out of the woods on this attack. Thank you for all the concern. I have just hung up with my primary care physician's referral team and let them know we need to plan for monthly Ivigg from now on. I do hope Cigna will not be too difficult to get on board. They were fine with every three months, but hopefully the presence of another attack will convince them of monthly treatment. The good news is that we were able to get blood samples drawn and sent to Dr. Druey. I hope he will get some good use of it. One doctor here at North Kansas City Hospital believes I should continue the theophylline along with the monthly ivigg. Any comments? I do not like the idea of filling my already flawed system with any unecessary drugs. If it is not needed in conjunction with Ivigg then I don't want to take it anymore. I pray for continued health for all of you and me. Cristina

In my case, I continued taking Theophylline for 5 months after going on a monthly regimen of IVIG, just in case -- even though we knew that the Theophylline was not preventing my episodes. In fact, they were coming more and more frequently. But then, we knew a lot less about the effectiveness of IVIG in those days.

My prayers are with you Christina. Your picture looks like you have small children. Makes this disease even more difficult. This disease peals back soooo many layers on the family! So I pray you will be home very soon. I have had this disease since October 2004. Mayo Clinic prescribed terbutaline and Theo-24. December of 2010 I had three very severe episodes. Hospital would always give me steroids, etc. was not until the third episode they gave me IVIG which helped immediately! I had a port put in, tried to start fusions, I did 3 months of them and then had huge problem with swelling in my head!! Like a monster! I always say there is a monster in me and I want him to Flee!!!:) They finally discovered that I had scar tissue in vein where port line was. They had to take out port and balloon vein. Much better now. This past July had another bad attack! Tried to get sample to Dr. Druey, for some reason or another it did not happen. So I am soo very happy you were able to send one to him. He is an awesome doctor and I am praying he will find a cure for us all!! I have now started monthly fusions again, getting ready for my 5th one in row!!! I still have to go to the vascular center first. They have to place a pick line, and it has to be done by a surgeon because I am so difficult. I believe it is due to the attacks have severely compromised my vascular system. I have it placed for two days until my fusion is done and then they take it out! Not fun, but much better than the alternative. Each month I am noticing strength renewed. It is amazing how it works. Yes we are all alike and yet unique in our struggles with this disease. I wish you and your family a very Merry Christmas and that you will be home! Christmas of 2010 was the morning of one of my attacks, went in hsp Christmas morning and did not come home until New Years Day!! Praying this Christmas and New Years we will all be home with our family and loved ones embracing the new year and each other!! Best of health to you, God Bless! Mary Jane

Hi Cristina. My husband still takes Theophylline even though he's on monthly IVIG. He is considering stopping it for the same reasons you want to stop, it's an extra drug in his system and there seems to be no benefit. However, he's reluctant to because his condition isn't really in full remission. It's a difficult choice to make.

I have not taken any Theophylline in the last five months. After being on 900 mg per/day, and 25 mg Terbutaline per/day for the last 8 years! I have not taken Terbutaline as well. The last five months I am doing IVIG and doing well without all those drugs in my system. I still had attacks while on those drugs anyway, and now while on IVIG nothing! Just sharing my experience, hope it helps. Are we truly ever in full remission?? Take care.

hi every boudy as well I have not taken any Theophylline since i hade dianosed by dr GREEB AND DR AMOURA i start with ivig with 2 g/kg and in the last 2 years i us 1 g/kg i have not any atak every thing is gioing well thnkes for good i hope that alsow for all of you,that what was recomanded by dr Greeb and dr Amoura yaser

Hi ! I stopped taking the theophyline & Terbutaline 1 month after I began my IVIG. My PCP wanted my to stay on for 6 months, but I just couldnt stand the side effects any more. My boys were 5 & 3 during my 1st major attack in 2000. They have adjusted well & I must brag a little. They both have a deep compassion for everyone-which I credit this "crazy" illness for. They grew up going to PT with me, the wheelchairs, prothestics, & me doing everyday things differently than other Moms. Twelve years later I still hear "Mom, I'm so glad you didnt die-I couldnt imagine growing up without you" Hang in there - God Bless.

Same story me theophyline & Terbutaline did not work and had 4 attacks whilst on them. So after a few month ofter starting IVIG I stopped taking them. Great to hear you got some samples to Dr Druey

Hello everybody, I am a french woman who is living near Paris. Like you I suffer of SCLS ,my first attack was in 2009 ,the seconde in 2012 without knowing what it was ,and the last one was the last december. During the last one I got to the hospital and doctors diagnosed this syndrome. I am going to go back to the hospital the 11th of february,after that I will have a treatment I think IVIG to. I didn't think it was possible to have this kind of disease !!! I am glade to have found your forum, in France It doesn't exist because we are not enough......with you, I am hopeful!!!!

Many of your questions will be answered as you read the different Community topics. Welcome aboard.

Christina So glad that you are doing better. I would be interested to understand if your doctors recommend a change to your current regimen. Like you, my attacks can be several years apart and the doctors are undecided if I require IVIG and if so how often. I am visiting NIH for the third time to conduct more tests for further evaluation. Good Luck in your continued recover. Robert

This condition affects people worldwide. Arturo and the others that founded this scls site on rareshare and the rest of us that participate know know that there is strength in numbers. Sharing experiences helps us grow in knowledge, helps others become aware of what scls is and provides an opportunity to connect with others whose lives has been affected with this rare condition. Best of luck with your journey- much progress has been made.

thank you for your reply. I wanted to know if some one of you can develop a myelome ? I will have every 6 months a test for this...what a disease !!!!

I have forgotten to ask you : do you know how many people in the USA are touched by this disease ?how many people die per year? In France it seems we are about 60 people . For the moment I feel alone with my disease, I am followed in Paris, doctors knew it they said me my disease only after 4 days of tests.

Starfrance My understanding is that the MGUS protein is associated with an increased chance of a myelome. I get the levels of MGUS checked frequently to see if there are any changes in its levels. You are actually quite lucky as there appears to be far more know and awarenace of SCLS in France than here in the UK. As far as I know there are only 2 in the UK and absolutely nobody has heard of it. As others have made you aware there is a centre which deals with SLCS in Paris. Good luck.

Hi all, I have been on monthly IVIG now for 3 months. After being discharged from the hospital in November insurance is on board for monthly treatments. I stopped taking theophylline and seem to be doing fine without it. My treatments are now every two weeks with the first treatment of the month being 30g and the second treatment is 35g. I have not had any symptoms since being released in Nov. I pray this is the answer, but I am always being vigilant with how I feel. Germs are unavoidable especially with 3 of my four children at home bringing everything they are exposed to at school home with them. Take care, Cristina

Hi Cristina It is good to hear the ivig is working!! Hopefully it will give you some security and peace. I saw what you wrote about the hemocue and while it would also provide you peace of mind I think that if the ivig continues to work for you that the hemocue nay become less important. If your episodes return while on ivig then it becomes more of an urgent need. In the meantime a blood pressure cuff at home will help you moniter any severe drop in your blood pressure and if in doubt seek professional care. The biggest insurance battle used to be getting the ivig so it is good that is not as much of an issue these days. As far as germs from what I understand the ivig gives your immune system a boost so hopefully that also will be less of a concern. My brother who has scls lives in the washington dc area, takes the metro everyday, travels to places like Indonesia and thailand so has been exposed to germs worldwide. He hydrates, takes vitamins and rests if he gets run down but he does not avoid interaction or activities because of germs. I know what has happened to you is terrifying and you are doing a great job handling it by being proactive and informed but don't let it keep you from enjoying life. For a mom kisses and hugs from your kids are the best medicine! I wish you the best! Linda